Newman Endo Repro Flashcards

1
Q

List primary symtpoms primary adrenal insuffiency

A

Ok

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What space are kidneys and adrenals

A

Retroperitoneum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Short term stress

A

Catecholamines, NE, E

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Long term stree

A

Glucocorticoids mineralocorticoids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

GFR

A

Glomerulosclerosis, fasciculata, reticularis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Salty sweet spiced

A

Mineralocorticoids, glucocorticoids, sex steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Describe HPA axis cortisol

A

Hypothalamus release CRH to anterior pituitary

Anterior pituitary send acth to adrenal cortex

Adrenal cortex makes cortisol which sends negative feedback to anterior pituitary and hypothalamus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

RAAS

A

Renin angiotensin aldosterone system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Aldosterone effect

A

Na retention

K secretion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Baby in looked good in nursery and feeding ok. Comes in day 6 thrown up but looks dehydrated, very dry mucous membranes(signs in baby—dry mucous membranes, no tears, anterior fontanells) bp 60/30, sluggish weak cry

Find a female genetalia, looks like a penis? Masculine , clitoramegonly, maasculinzation of female genetalia
VIRILIZED

A

Virilized, low bp, dehydration

21 hydroxylase defiency
Congenital adrenal hyperplasia

Lack of aldosterone

Lack of cortisol and aldosterone…flow over to DHEA< androstenedione, and testerostone

Lose na lose water

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Lab work CAH

A

Na low, K high, Cl low, bicarb low, BUN high, glucose low

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Why girl better CAH

A

Girl genital show virilization

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Why glucose low

A

Cortisol stimulate gluconeogenesis (something insulin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What order

A

17 a OH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Treat CAH

A

Hydroxycortisone, fluids/glucose, get K level down

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Primary adrenal insuffiency

A

Autoimmune (most common cause in US)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Most common infectious cause primary adrenal insuffiency

A

TB-SEE CALCIFICATIONS ON MRI

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

X linked recessive disorder of long chain FA metabolism

A

Mimic MS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Waterhouse friedreich Sean syndrome

A

Bilateral adrenal hemorrhage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

ACTH darkening of the skin

A

MSH of PMC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Inherited adrenal unresponsiveness to ACTH

A

Familial glucocorticoid defiency (AR)

Increase acth causes skin pigmentation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Triple A

A

Rare AR adrenal gland no respond to ACTH so ACTH so high

Mutation int he AAAAS gene that codes for the protein ALADIN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Congenital adrenal hyperplasia

A

21 hydroxylase defiency most common

Salt wasting

Defiency cortisol and aldosterone

Excessive androgens

Sometimes called Addison in older children/adulthood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Adrenal insuffiency how do they present

A

Fatigue, reduced stamina, weakness, anorexia, weight loss, skin hyperpigmentation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What do patients complain of with primary adrenal insuffiency

A

Abdominal pain, nausea, vomiting, msk pain (joint, muscles, chest, back), psychiatric (depression, anxiety, irritability), HA, vomiting, gait disturbance, salt craving and orthostatic light headedness, BP is usually low and orthostatic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Female infant with 21 hydroxylase defiency will have virilized genetalia

A

Ok

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Labs primary adrenal insuffiency

A

Na low, K high, low blood sugar, maybe hypercalcemia, plasma cortisol ***

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Low 8 am plasma cortisol (low morning high night) and do ACTH level and its high

A

Primary adrenal insuffiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Confirmatory test

A

Cosyntropin (synthetic acth) stimulation test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

If cortisol doesn’t respond to acth

A

Confirm primary corticoinsuffiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Serum antibodies to 21 hydroxylase

A

Confirm autoimmmune Addison

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Lab to see 21 hydroxylase defiency

A

Elevated serum levels of 17 oh progesterone are required

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Plasma renin high primary adrenal insuffiency

A

Sense hypovolemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Treat primary adrenal insuffiency

A

Glucocorticoids replacement therapy, mineralocorticoids therapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Acute adrenal crisis symptoms

A

Ab pain

Confusion

Dehydration

Dizziness

Fatigue

High fever (

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Labs acute adrenal crisis

A

ACTH stimulation test, cortisol blood sugar, K , serum Na, serum pH (

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Secondary adrenal insuffiency

A

Low serum ACTH in the setting of low serum cortisol is the give away

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

32 year old purple tiger stripes on abdomen , high BP, periods irregular, crappy feeling, hump back

A

Striae, Cushing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Cushing

A

Excessive corticosteroids

Vague weird symptoms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

ACTH dependent cushing

A

Cushing disease (ACTH secretory adenoma)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

ACTH independent cushing syndrome

A

Exogenous corticosteroid use (most common cause)

Adrenocortical tumor

42
Q

Mccune albright syndrome

A

Polycyotic fibrous dysplasia, cafe at lair, endocrine hyperfunction from multiple organ)

43
Q

Extopic acth

A

AAC of lung

44
Q

Cushing disease

A

Pituitary adenoma

45
Q

Cushing syndrome

A

Ectopic acth

SSC of lung

46
Q

Dexamasone test

A

Draw serum acth before

47
Q

ACTH lelvated in hundred, cortisol not suppressed by high or low doses

A

Ectopic acth syndrome is likely

48
Q

Undetectable or low acth, cortisol not suppressed by high or low doses

A

Primary hypercortisolism is likely (not driven by acth, possible adrenal tumor)

49
Q

Normal to elevated acth, cortisol not suppressed by low but suppressed by IgG

A

Cushing disease (pituitary still retains some feedback control)

50
Q

Incidentalomas

A

Accidentally find nodule in adrenal >4 cm have to do work up and makes sure not malignant with CT and MRI

51
Q

All patients with adrenal incidentaloma require testing for _____. How

A

Pheochromocytoma

Plasma metanephrines

Urine collection

52
Q

Primary aldosteronism

A

HTN that is resistant and descent respond to three drugs think of this

53
Q

Conn syndrome

A

Primary aldosteronism secondary to a unilateral aldosterone-producing adrenal adenoma

40% have a mutation in a gene coding for the K channel

54
Q

Signs symtpoms primary aldosteronism

A

HTN and hypokalemia

55
Q

Lab finding primary aldosteronism

A

Metabolic alkalosis (proton lows, H with K)

56
Q

Pheochromocytoma

A

VHL type 2
AD
Get it

Bp high, sweating,

57
Q

Lab pheochromocytoma

A

Plasma fractioned free metanephrines is the single msot sensitive test for secretory pheochromocytoma and paragangliomas

58
Q

Treating suspected pheochromocytoma

A

Alpha blocker before beta blocker otherwise bp can go up

59
Q

MEN

A

AD

Have tumours in 2 or more different endocrine glands

60
Q

Growth curve PEDS : how measure length

A

Laying down

61
Q

Measure height or stature

A

Standing up

62
Q

Over 36 months

A

Standing up

63
Q

Normal BMI kids

A

Percentile

64
Q

Normal weight kids

A

Greater than or equal to 5th percentile and less than 85th percentile

65
Q

Normal weight adult

A

BMI 18,5-24.9

66
Q

6 here’s of age till puberty growth per year

A

2 inches a year

67
Q

Mid parental height

A

Genetic potential …estimate of genetic potential for girls and boys different

68
Q

Weight falls off first

A

Think nutrition

Not getting enough or processing enough

Higher than average caloric requirements

69
Q

Length fallow off first

A

Think endocrine

GH defiency, hypothyroidism, cushing syndrome (iatrogenic)

70
Q

Head circumference falls off first

A

Primary failure of the brain to grow

Severe craniosynstosis

71
Q

Bone age

A

Provides a rough estimate of a kids skeletal maturation by assessing the ossification of the epiphyseal centers

Bone age is 2 SD below the chronological age is considered delayed

72
Q

What bone age is considered delayed

A

2 SD

73
Q

Abnormal growth

A

A comprehensive history and physical is essential in developing the differential diagnosis and guiding the work up

74
Q

What most important of work up of kid not growing well

A

History and physical

75
Q

Evaluating growth. How many points on the growth curve

A

More better

76
Q

Get as many points on growth curve as you can

A

Ok

77
Q

Catch up or catch down

A

First 18 months of life plotting kids make 25% downward adjustment as go from Abby food to solid food. They’ll catch p but a lot make a downward adjustment.

78
Q

Constitutional growth delay

A

Late bloomer, few months big growth

Grow grow grow, slow down then catch back up

79
Q

End height if have precocious puberty

A

Shorter than normal. Growth plates mature cant grow anymore

Grow really fast when dont expect then off

Familial -come form land of little people

80
Q

Cushing curve

A

Gaining weight, but height not up up

81
Q

Short stature

A

Height 2SD less than the 3rd percentile (mean height for age and sex)

Height more than 2SD below the mid parental height

82
Q

Intima work up for concerning short stature and not explained by constitutional, familial, and idiopathic

A

IGF and IGFBP-3 levels

83
Q

IGF and IGFBP-3 levels

A

Insulin like growth factor

84
Q

Turner

A

45 XO with delayed puberty/short stature in females always check a karyotyoe

Web neck
Wide nipples

85
Q

Lab work turner0short stature

A

Check karyotype and see if 45 XO

86
Q

Precocious puberty less than 8 years old girls and 9 in men

A

Full activation of HPG axis before 8 years of age in girls and before 9 years of age in boys

87
Q

Consider precocious puberty in girls

A

Progressive breast development

Crossing major percentile lines upward on the linear growth chart

88
Q

Consider precocious puberty in boys if

A

Evidence of both testicular and penile enlargement

Crossing major percentile lines upward not he linear growth chart

89
Q

Bone age precocious puberty

A

Advanced bone age

90
Q

Premature adrenarche

A

Increase in DHEA and its storage from DHEA-S

Pubic hair, axillary hair ,odor, sometimes acne

91
Q

Straight thin hair

A

No worry

92
Q

Curly thick hair and other secondary sexual characteristics of growth sky rocketing

A

Work up look at growth look at other sexual characteristics

93
Q

Only thin little pubic hair

A

Fine

94
Q

Premature thelarche

A

Breast early

Usually feel bud in kids just watch

If related to acceleration work up as precocious puberty

95
Q

Hypothyroidism

A

Increase BMI from growth arrest with continued weight gain

Family history always watch

96
Q

Tall stature

A

Beckwith-wiedemann syndrome(big head wen young, normal adult height)
Insulin like growth factor 2

97
Q

Homocystinuria

A

Infant of diabetic mother

Big when little

98
Q

Klinefelter syndrome

A

47 XXY

99
Q

Marian

A

AD
Connective tissue

Fibrillin-1 gene mutation genetic consultation

Dissecting aortic aneurysm

100
Q

When refer growth

A

Less than 3rd percentile or greater 95th and continues to rise or drop

Increase growth velocity between schoool age and puberty

Growth potential: projected height varies from midparental height by more than 2 inches

Multiple dystrophic features send to geneticist

Bone age advanced of delayed by more the 2 SF